340 research outputs found

    Multiple Sclerosis in Children

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    How to Cite This Article: Inaloo S, Haghbin S. Multiple Sclerosis in Children. Iran J Child Neurol. 2013 Spring;7(2):1-10. Multiple sclerosis (MS) is the most important immune-mediated demyelinated disease of human which is typically the disease of young adults. A total of 4% to 5% of MS population are pediatric. Pediatric MS is defined as the appearance of MS before the age of sixteen. About 80% of the pediatric cases and nearly all adolescent onset patients present with attacks typical to adult MS. Approximately 97% to 99% of the affected children have relapsing-remitting MS, while 85% to 95% of the adults experience such condition. MS in children is associated with more frequent and severe relapses. Treatment is the same as adults. We aimed to review the epidemiology, etiology, clinical manifestations, and treatment of MS in children. References1. Lublin F. History of modern multiple sclerosis therapy. J Neurol 2005 Sep;252(Suppl 3):iii3-iii9. Review.2. Murray TJ. Robert Carswell: the first illustrator of MS. Int MS J 2009 Sep;16(3):98-101.3. Kabat EA, Glusman M, Knaub V. Quantitative estimation of the albumin and gamma globulin in normal and pathologic cerebrospinal fluid by immunochemical methods. Am J Med 1948 May;4(5):653-62.4. Kumar DR, Aslinia F, Yale SH, Mazza JJ. Jean-Martin Charcot: the father of neurology. Clin Med Res 2011 Mar;9(1):46-9.5. Dawson JD. The histology of disseminated sclerosis.Trans of the Roy Soc Edinb. 1916;50:517-740.6. Gadoth N. Multiple sclerosis in children. Brain Dev 2003 Jun;25(4):229-32. Review.7. Banwell BL. Pediatric multiple sclerosis. Curr Neurol Neurosci Rep 2004 May;4(3):245-52.8. Renoux C, Vukusic S, Mikaeloff Y, Edan G, Clanet M, Dubois B, et al. Natural history of multiple sclerosis with childhood onset. N Engl J Med 2007 Jun 21;356(25):2603-13.9. Boiko A, Vorobeychicle G, Paty D, Devonshire V, Sondovnick D. Early onset multiple sclerosis: a long longitudinal study. Neurology 2002 Oct 8;59(7):1006-10.10. Yavari MJ, Inaloo S, Saboori S. Multiple sclerosis in children: A review of clinical and paraclinical features in26 cases. Iran J Child Neurol 2008;2(4):41-46.11. Oksenberg JR, Baranzini SE, Sawcer S, Hauser SL. The genetics of multiple sclerosis: SNPs to pathways to pathogenesis. Nat Rev Genet 2008 Jul;9(7):516-26.12. Willer CJ, Dyment DA, Risch NJ, Sadovnick AD, Ebers GC; Canadian Collaborative Study Group. Twin concordance and sibling recurrence rates in multiple sclerosis. Proc Natl Acad Sci USA 2003 Oct 28;100(22):12877-82.13. Ramagopalan SV, Knight JC, Ebers GC. Multiple sclerosis and the major histocompatibility complex. Curr Opin Neurol 2009 Jun;22(3):219-25.14. Banwell B, Krupp L, Kennedy J, Tellier R, Tenembaum S, Ness J, et al. Clinical features and viral serologies in children with multiple sclerosis: a multinational observational study. Lancet Neurol 2007 Sep;6(9):773-81.15. Alotaibi S, Kennedy J, Tellier R, Stephens D, Banwell B. Epstein Barr virus in pediatric multiple sclerosis. JAMA2004;291(15):1875-9.16. Pohl D, Knone B, Rostasy K, Kahler E, Brunner E, Lehnert M, et al. High seroprevalence of Epstein-Barr virus in children with multiple sclerosis. Neurology 2006 Dec12;67(11):2063-5.17. Waubant E, Mowry EM, Krupp L, Chitnis T, Yeh EA, Kuntz N,et al. Antibody response to common viruses and human leukocyte antigen-DRB1 in pediatric multiple sclerosis. Mult Scler. 2012 Dec 11.18. Waubant E, Mowry EM, Krupp L, Chitnis T, Yeh EA, Kuntz N, et al. Common viruses associated with lower pediatric multiple sclerosis risk. Neurology 2011 Jun 7;76(23):1989-95.19. Mikaeloff Y, Caridade G, Rossier M, Suissa S, Tardieu M. Hepatitis B vaccination and the risk of childhoodonsetmultiple sclerosis. Arch Pediatric Adolesc Med 2007;161:1176-82.20. Hammord SR, English DR, Moleod JG. The age-range of risk of developing multiple sclerosis. Brain. 2000 May;123 (Pt 5):968-74.21. Van Amerongen BM, Dijkstra CD, Lips P, Polman CH. Multiple sclerosis and vitamin D: an update. Eur J Clin Nutr 2004 Aug; 58:1095-109. 22. Willer CJ, Dyment DA, Sadovnick AD, Rothwell PM, Murray TJ, Ebers GC, et al. Timing of birth and risk of multiple sclerosis: population based study. BMJ 2005 Jan;330(7):120.23. Mowry EM, Krupp LB, Milazzo M, Chabas D, Strober JB, Bellman AL, et al. Vitamin D status is associated with relapse rate in pediatric-onset multiple sclerosis. Ann Neurol 2010 May;67(5):618-24.24. Banwell B, Bar-Or A, Arnold DL, Sadovnick D, Narayanan S, Mc Gowan M, et al. Clinical, environmental, and genetic determinants of multiple sclerosis in children with acute demyelination: a prospective national cohort study. Lnacet Neurol 2011 May;10(5):436-45.25. Disanto G, Morahan JM, Ramagopalan SV. Multiple sclerosis: risk factors and their interactions. CNS NeurolDisord Drug Targets. 2012 Aug;11(5):545-55. 26. Munger KL, Chitnis T, Ascherio A. Body size and risk of MS in two cohorts of US women. Neuroloty 2009 Nov 10;73(19):1543-50.27. Renoux C, Vukusic S, Mikaeloff Y, Edan G, Clanet M, Dubois B, et al. Natural history of multiple sclerosis withchildhood onset. N Engl J Med 2007 Jun; 356(25):2603-13.28. Gusev E, Boiko A, Bikova O, Maslova O, Guseva M, Boiko S, et al. The natural history of early onset multiple sclerosis: comparison of data from Moscow and Vancouver. Clin Neurol Neurosurg 2002 Jul;104(3):203-7.29. Simone IL, Carrara D, Tortorella C, Liquori M, Lepore V, Pellegrini F, et al. Course and prognosis in early-onsetMS: comparison with adult-onset forms. Neurology 2002 Dec;59(12):1922-8.30. McDonald WI, Compston A, Edan G, Goodkin D, Hartung HP, Lublin FD, et al. Recommended diagnostic criteria for multiple sclerosis: guidelines from the International Panel on the diagnosis of multiple sclerosis. Ann Neurol 2001 Jul; 50(1):121-7.31. Polman CH, Reingold SC, Edan G, Filippi M, Hartung HP, Kappos L, et al. Diagnostic criteria for multiple sclerosis: 2005 revisions to the “McDonald Criteria”. Ann Neurol 2005 Dec;58:840-6.32. Swanton JK, Rovira A, Tintore M, Altmann DR, Barkhof F, et al. MRI criteria for multiple sclerosis in patients presenting with clinically isolated syndromes: a multicentre retrospective study. Lancet Neurol 2007 Aug;6(8):677-86.33. Rovira A, Swanton J, Tintore M, Sastre-Garriga J, Horga A, et al. A single, early magnetic resonance imaging study in the diagnosis of multiple sclerosis. Arch Neurol 2009 May;66(5):587-92.34. Poser CM, Paty DW, Scheinberg L, McDonald WI, Davis FA, Ebers GC, et al. New diagnostic criteria for multiple sclerosis: guidelines for research protocols. Ann Neurol 1983 Mar;13(3):227-31.35. Polman CH, Reingold SC, Banwell B, et al. Diagnostic criteria for multiple sclerosis: 2010 revisions to the McDonald criteria. Ann Neurol 2011 Feb; 69(2):292-302.36. Mikaeloff Y, Adamsbaum C. Husson B, Vallee L, Ponsot G, Confavreux C. et al. MRI prognostic factors for relapse after acute CNS inflammatory demyelination in childhood. Brain 2004 Sep;127(Pt9):1942-7.37. Chabas D, Castillo-Trivino T, Mowry EM, Strober JB, Glenn OA, Woubant E, et al. Vanishing MS T2-bright lesions before puberty: a distinct MRI phenotype? Neurology 2008 Sep;71(14):1090-3.38. Krupp LB, Banwell B, Tenembaum S. Consensus definitions proposed for pediatric multiple sclerosis andrelated disorder. Neurology 2007 Apr;68(16 Suppl 2):S7-S12.39. Yeh EA, Chitnis T, Krupp L, Ness J, Chabas D, Kuntz N, et al. Pediatric multiple sclerosis. Nat Rev Neurol 2009 Nov;5(11):621-31.40. Banwell B, Ghezzi A, Bar-Or A, Mikaeloff Y, Tardien M. Multiple sclerosis in children: clinical diagnosis, therapeutic strategies, and future directions. Lancet Neurol 2007 Oct;6(10):887-902.41. Venkateswaran S, Banwell B. Pediatric multiple sclerosis. Neurologist 2010 Mar;16(2):92-105.42. Waubant E, Chabas D, Okuda DT, Glenn O, Mowry E, Henry RG, et al. Difference in disease burden and activity in pediatric patients on brain magnetic resonance imaging at time of multiple sclerosis onset vs adults. Arch Neurol 2009 Aug; 66(8):967-71.43. Ghassemi R, Antel SB, Narayanan S, Francis J, Bar-or A, Sadovnick AD, et al. Lesion distribution in children with clinically isolated syndromes. Aim Neurol 2008 Mar;63(3);401-5.44. Yeh EA, Weinstock-Guttman B, Ramanathan M, Ramasamy DP, Willis L, Cox JL, et al. Magnetic resonance imaging characteristics of children and adults with paediatric-onset multiple sclerosis. Brain 20 Dec;132:3392-400.45. Mikaeloff Y, Suissa S, Vallee L, Lubetzki C, Ponsot G, Confavreux C, et al. First episode of acute CNS inflammatory demyelination in childhood: prognostic factors for multiple sclerosis and disability. J Pediatr 2004 Feb;144(2):246-52.46. Chabas D, Ness J, Belman A, Yeh EA, Kuntz N, Gorman MP, et al. Younger children with MS have a distinct CSF inflammatory profile at disease onset. Neurology 2010 Feb 2;74(5):399-405.47. Gronseth GS, Ashman U. Practice parameter: the usefulness of evoked potentials in identifying clinicallysilent lesions in patients with suspected multiple sclerosis (an evidence-based review): Report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 2000 May 9;54(9):11720-5.48. Boutin B, Esquivel E, Mayer M, Chaumet S, Ponsot G, Arthuis M, et al. Multiple sclerosis in children: report of clinical and paraclinical features of 19 cases. Neuropediatrics 1988 Aug;19(3):118-23.49. Waldman AT, Gorman MP, Rensel MR, Austin TE, Hertz NL. Management of pediatric central nervous system demyelinating disorders: consensus of United States neurologists. J Child Neurol 2011 Jun;26(6):675-82.50. Banwell BL. Pediatric multiple sclerosis. Curr Neurol Neurosci Rep 2004 May;4(3):245-52. 51. Yeh EA, Weinstock-Guttman B. The management of pediatric multiple sclerosis. J Child Neurol 2012;27:1384-1393.52. Ghezzi A, Amato MP, Capobianco M, Gallo P, Marrosu G, Matinelli V, et al. Disease-modifying drugs in childhood-juvenile multiple sclerosis: results of an Italian co-operative study. Mult Scler 2005 Aug;11(4):420-4.53. Banwell B, Reder AT, Krupp L, Tenembaum S, Eraksoy M, Alexy B, et al. Safety and tolerability of interferon beta-1 b in pediatric multiple sclerosis. Neurology 2006 Feb;66(4):472-6.54. Tenembaum SN, Segura MJ. Interferon beta-la treatment in childhood and juvenile-onset multiple sclerosis. Neurology 2006 Aug 8;67(3):511-3.55. Pohl D, Waubant E, Banwell B, Chabas D, Chitnis T, Weinstock-Guttman B, et al. Treatment of pediatric multiple sclerosis and variants. Neurology 2007 Apr;68(16 suppl):S54-65.56. Makhani N, Gorman MP, Branson HM, Stazzone L, Banwell BL, Chitnis T, et al. Cyclophosphamide therapy in pediatric multiple sclerosis. Neurology 2009 Jun;72(24):2076-80.57. Goodin DS, Amason BG, Coyle PK, Frohman EM, Paty DW, et al. The use of mitoxantrone (Novantrone) for the treatment of multiple sclerosis: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology 2003 Nov 25;61(10):1332-8.58. Ghezzi A, Pozzilli C, Grimaldi LM, Brescia Morra V, Bartolon F, Capra R, et al. Safety and efficacy of natalizumab in children with multiple sclerosis. Neurology 2010 Sep ;75(10):912-7.59. Mancordi GL, Saccardi R. Autologous heamatopoietic stem cell transplantation in multiple sclerosis. Lancet Neurol 2008 7:626-636.60. Attarian HP, Brown KM, Duntley SP, Carter JD, Cross AH, et al. The relationship of sleep disturbances and fatigue in multiple sclerosis. Arch Neurol 2004 Apr;61(4):525-30.61. Krupp LB, Alvarez LA, LaRocca NG, Scheinberg LC. Fatigue in multiple sclerosis. Arch Neurol 1988 Apr;45(4):435-7.62. MacAllister WS, Belman AL, Milazzo M, Weisbrot DM, Christodoulou C, Scheri WF, et al. Cognitive functioningin children and adolescents with multiple sclerosis. Neurology 2005 Apr;64(8):1422-5

    Febrile Seizure in Thalassemic Patients

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    ObjectiveFebrile seizure is the most common seizure disorder in children. Its pathophysiology is not fully understood yet; however, some risk factors have been cited for it. Iron is one of these influential elements and is involved in the metabolism of some neurotransmitters which are reduced in irondeficiency anemia and also increases the sensitivity of neural cells during a febrile episode. The present study aimed to determine the rate of febrile seizure in thalassemic patients and to compare it with the corresponding rate in the normal population.Materials & MethodsThis descriptive cross-sectional study was conducted on 766 patients with thalassemia major. They were all older than 6 months and were referred to Dastghaib Cooly's Clinic, affiliated to Shiraz University of Medical Sciences, from Oct 2006 to May 2007, and 766 normal and healthy children as the control group. Questionnaires containing demographic data and past history of febrile seizure, age of febrile seizure, number of episodes, hospitalization, and related family history were prepared and filled through interviewing the parents.ResultsFebrile seizure was detected in 7 cases of the patient group (0.9%) versus 18 cases (2.3%) of the control group. The frequency of febrile seizure in the controls was 2.5 times more than that in the thalassemia group, which was statistically significant (P < 0.05).ConclusionThis study showed a lower rate of febrile convulsion in thalassemic patients compared to the control group. Accordingly, it could be suggested that high iron storage is a protective factor against febrile convulsion.

    An Isolated Integrated Charger for Electric or Plug-in Hybrid Vehicles

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    For electric and hybrid vehicles using grid power to charge the battery, traction circuit components are not normally engaged during the charging time, so there is a possibility to use them in the charger circuit to have an on-board integrated charger.In this Licentiate thesis, an isolated high power integrated charger is proposed, designed and constructed based on a special ac machine with a double set of stator windings called motor/generator.The charger is capable of unit power factor operation as well as bi-directional power operation for grid to vehicle application.The mathematical electromechanical model of the motor/generator is derived and presented. Based on the developed model, new controller schemes are developed and designed for the grid synchronization and charge control. The machine windings are re-arranged for the traction and charging by a controllable relay-based switching device that is designed for this purpose.A laboratory system is designed and implemented based on a 44 pole 25 kW25~kW interior permanent magnet synchronous motor and a frequency converter considering the integrated charging features for winding re-configuration. The practical results will be added in the next step of the project. The charging power is limited to 12.5 kW12.5~kW due to the machine thermal limit (half of the motor full power in the traction mode) for this system.The whole system is simulated in Matlab/Simulink based on the developed model and controllers to verify the system operation for the charge control. Simulation results show that the system has good performance during the charging time for a load step change. The simulation results show also a good performance of the controllers leading to machine speed stability and smooth grid synchronization. Moreover, the unit power factor operation is achieved for battery charging in the simulations

    Frozen Leg Operation of a Three-Phase Dual Active Bridge Converter

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    An Isolated Integrated Charger for Electric or Plug-in Hybrid Vehicles

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    For electric and hybrid vehicles using grid power to charge the battery, traction circuit components are not normally engaged during the charging time, so there is a possibility to use them in the charger circuit to have an on-board integrated charger.In this Licentiate thesis, an isolated high power integrated charger is proposed, designed and constructed based on a special ac machine with a double set of stator windings called motor/generator.The charger is capable of unit power factor operation as well as bi-directional power operation for grid to vehicle application.The mathematical electromechanical model of the motor/generator is derived and presented. Based on the developed model, new controller schemes are developed and designed for the grid synchronization and charge control. The machine windings are re-arranged for the traction and charging by a controllable relay-based switching device that is designed for this purpose.A laboratory system is designed and implemented based on a 44 pole 25 kW25~kW interior permanent magnet synchronous motor and a frequency converter considering the integrated charging features for winding re-configuration. The practical results will be added in the next step of the project. The charging power is limited to 12.5 kW12.5~kW due to the machine thermal limit (half of the motor full power in the traction mode) for this system.The whole system is simulated in Matlab/Simulink based on the developed model and controllers to verify the system operation for the charge control. Simulation results show that the system has good performance during the charging time for a load step change. The simulation results show also a good performance of the controllers leading to machine speed stability and smooth grid synchronization. Moreover, the unit power factor operation is achieved for battery charging in the simulations
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